Renal amyloidosis in a child with chronic granulomatous disease and invasive aspergillosis.

A boy who had been diagnosed with chronic granulomatous disease (CGD) at the age of 6.5 years had a medical history of multiple bacterial infections, including pneumonia, staphylococcal liver abscesses and septicemia, from birth. At the age of 10 years and 4 months he developed an infection that was accompanied by high fever and pulmonary, mediastinal and paravertebral infiltrations. Aspergillus niger was cultured on bronchial secretions obtained by bronchoscopy. Shortly thereafter, proteinuria manifested and progressed to the nephrotic level. A skin biopsy indicated a diagnosis of amyloidosis. An anti-fungal treatment with amphotericin B and other agents, along with surgical pus drainage, intravenous leukocyte mass, interferon-gamma and immunoglobulin infusions, was ineffective, and the patient eventually died from multi-organ failure. The postmortem examination revealed the presence of disseminated aspergillosis and systemic amyloidosis. Although no direct evidence is available that would confirm the causative role of aspergillosis in the development of systemic amyloidosis, to the best of our knowledge this is the first report of a CGD case with complications of both invasive aspergillosis and systemic amyloidosis.

[Congenital respiratory cystic dysplasias in children]. / Vaiku kvepavimo trakto igimtos cistines displazijos.

UNLABELLED: Diagnosis and treatment of congenital respiratory cystic dysplasias - more complicated than acquired pulmonary diseases. In 20% of cases treatment should be started in newborn age because of respiratory distress syndrome. MATERIAL AND METHODS: During the period of 1990-2002 we have diagnosed and treated 30 patients due to congenital respiratory cystic dysplasias. Clinical symptoms, diagnostic methods, indications for the operation and results of the surgical treatment were analyzed. RESULTS: We operated on 18 boys and 12 girls. Age of the patients - 6 newborns, 8 infants, 3 children from 1 till 3 years, 13 patients from 7 till 15 years. Lobectomy was performed for 13 patients, segmentectomy in 1 case, and removal of the cyst in 14 cases. In 1 case videotoracoscopy and in 1 case angiography was made. All patients survived. Other diseases were found in 16 cases. CONCLUSIONS: Congenital respiratory cystic dysplasias are serious respiratory disease of newborns and children which can cause respiratory distress syndrome. 1. Because of respiratory distress, possible infection and malignant transformation surgical treatment is recommended. 2. Antenatal diagnosis of congenital respiratory cystic dysplasias could enable the neonatologist and surgeon to prepare for the treatment, because in 20% of cases the treatment is necessary in early postnatal period.

[Pulmonary sequestration]. / Plauciu sekvestracija.

UNLABELLED: Pulmonary sequestration is rare and serious dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. Pulmonary sequestration masses have autonomic blood supply. For confirmation of the diagnosis we use ultrasound examination, CT scan, MRI, angiopulmography and aortography. AIM OF THE STUDY: To present pathogenesis, diagnostic features and surgical treatment tactics of rare and serious pulmonary dysplasia. MATERIAL AND METHODS: During the period of 1979-2002, we have examined and treated 9 patients because of pulmonary sequestration. We analyzed clinical signs, results of the examination before operation, indications for operation and results of surgical treatment. RESULTS: Seven patients after the operation recovered, 1 patient died due to infection and cardiovascular complications. One patient is supposed to be operated on in the near future. CONCLUSIONS: 1. Pulmonary sequestration is rare and serious bronchial - pulmonary dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. 2. Pulmonary sequestrations are classified as intralobar, extralobar and extrapulmonary sequestration. 3. Pulmonary sequestration is often associated with cardiovascular dysplasias and diaphragmatic malformations. 4. Methods of examination: X-ray examination, CT scan, MRI, ultrasound examination, aortography. 5. Complications of pulmonary sequestration: pneumonia, pleuritis, necrosis of the sequestration, chylothorax, respiratory distress syndrome, hemothorax, cystic transformation, and pneumothorax. 6. Treatment - surgery or embolisation of abnormal supply vessels.